Mr. Speaker, it gives me great pleasure tonight to speak to Bill S-211, an act respecting national sickle cell awareness day.
The United Nations, the African Union, and the World Health Organization have all recognized sickle cell disease as a public health priority. They also all recognize June 19 of each year as World Sickle Cell Awareness Day. World Sickle Cell Awareness Day has been held on June 19 since 2008. That day was chosen to commemorate the day on which a resolution was officially adopted by the General Assembly of the United Nations. It recognizes sickle cell disease as a public health concern.
Quite honestly, until last September, I had heard about sickle cell disease but actually had no idea what it was or how serious it could be. I had the opportunity, though, to meet with members of the Sickle Cell Association of Canada, including my constituent in Saskatoon—Grasswood, Mr. Craig Eling. Craig has been a big help in preparing me for my remarks in the House today.
I would like to give some facts about this disease.
Sickle cell disease is the most common inherited blood-related disease. The World Health Organization, the WHO, estimates that up to 5% of the world's population are healthy carriers of the gene for sickle cell disease. It is most commonly found in people from sub-Saharian Africa, the Middle East, Mediterranean countries, and parts of India.
People get one gene from each of their parents. A person with one gene for sickle cell disease is known as a carrier, also known as sickle cell trait. Individuals live normal lives and are very rarely affected by their single gene, but here is where it becomes complicated. A person with two genes has sickle cell disease, also known as sickle cell anemia, and their lives are greatly impacted by this condition.
Sickle cell disease results in the production of abnormal red blood cells, which are shaped like a crescent moon or the letter "C." Normal red blood cells look like a doughnut without the hole. Sickle shaped blood cells like to get stuck because of the points on either end of the crescent moon shape, which causes many of the issues associated with the disease. The most common place they get stuck is in the organ that cleans the blood. That of course is the spleen. Because they get stuck in the spleen, it withers away by the time a child with sickle cell disease is about 5-years-old. Although people can live without their spleens, they are more at risk of certain types of blood-borne bacterial infections.
As soon as children are diagnosed with sickle cell disease, some as early as birth, they are put on daily antibiotics to prevent an infection. At an appropriate age, they will receive extra immunizations. This also means that the presence of a fever in a person with sickle cell disease could indicate a bacterial infection and such situations are considered a medical emergency that requires prompt treatment with antibiotics and sometimes even hospitalization.
A normal red blood cell lives in the body for about 120 days, while sickle cells live for about 10 to 20 days. The body is constantly destroying the sickle cells and working to produce fresh red blood cells. This can lead to chronic anemia, where the blood is unable to carry enough oxygen to all parts of the body. The common effects of chronic anemia are ongoing tiredness, pale skin, weakness, and delayed growth in development in children.
A crisis occurs when enough crescent moon shaped sickle cells jam up in a blood vessel, causing a disruption of normal blood flow. The body tissues downstream of the blockage lose oxygen and begin to die, which causes, in some cases, severe pain.
If not promptly treated, the loss of oxygen can lead to permanent damage to affected areas. The most serious and really the most relatively common outcome of sickle cell disease is a stroke. This can occur in children as young as two years old.
To prevent crisis, people with sickle cell disease must stay well hydrated and avoid rapid temperature changes. Children often cannot play outside in winter. They are also asked to avoid intensive exercise, eat a balanced diet, stay well rested, and avoid smoking.
They are required to take a special medication every day to decrease the amount of sickle cells the bone marrow produces and avoid crises. The only cure for sickle cell disease is a bone marrow transplant, which, by the way, does not occur very often because of a lack of an appropriate donor. Even though we have a bone marrow registry connected to worldwide donors, the best chances of an acceptable match would actually be a person's siblings.